Sickle Cell Disease: A Genetic Disorder Affecting Many Americans
Washington— Soon after Heaven Buchanan celebrated her 10th birthday this year, she was admitted into a Chicago hospital, where she would stay for almost three weeks with severe pain in her arms and legs.
The pain is all too familiar for Heaven, who enjoys playing with Barbies, jamming to Beyonce's latest hits and making digital art. She is one of the approximately 100,000 Americans dealing with sickle cell disease, a genetic disorder affecting the shape of the red blood cells that can lead to blood flow complications and a range of other painful issues.
"It was very concerning because her oxygen level stayed low," Heaven's mother, Levella Golden, said about the hospital stay, which included blood transfusions, high-dose pain medicine, and supplemental oxygen. "I felt bad as a parent, just seeing her lying like that, and there [was] nothing I [could] do."
Black Americans like Heaven represent the overwhelming majority of those living with sickle cell disease. Approximately 1 in 365 African American children are born with sickle cell, according to the Centers for Disease Control and Prevention.
Children with Sickle Cell Disease Face Financial Burden
When Heaven is in the hospital or has to stay home from school, her mother often has to take time off from the maintenance job that she has had for seven years. But that can make it hard to pay for food or put gas in the car for Heaven's various doctor's appointments, an added stress on top of her daughter's pain.
New federal data obtained by CBS News, as well as interviews with experts, advocates, patients and policymakers, show how one safety net program meant to alleviate that stress has been out of reach for thousands of children who need it most.
"Completely mystified"
Across the country, many families with a child diagnosed with sickle cell disease told CBS News they face a financial predicament like Heaven's mom. Watching over a child in the hospital or staying home to administer pain medication means missing out on paychecks. While Heaven's medical care is covered by Medicaid, the family still racks up related costs, like over-the-counter medicines and therapies.
The federal government has programs meant to alleviate the financial burden that often accompanies long-term medical issues like sickle cell disease among children, including disability payments from a program run by the Social Security Administration, or SSA. The program is known as Supplemental Security Income, or SSI.
In 1990, there was a significant Supreme Court case that relaxed the restrictions on determining when children were considered "disabled." This change expanded the eligibility for disability payments for children, aligning it with the regulations for adults. As a result, more than half of the disabled child applicants were approved for assistance, which was almost three times the previous number, according to the National Center for Youth Law.
Social Security disability payments are intended for low-income Americans and can reach up to $914 per month. However, the actual payments are typically lower and depend on factors such as income and household circumstances.
Dr. Andrew Campbell, the director of the Comprehensive Sickle Cell Disease Program at Children's National Hospital, emphasized that the money can assist families who are "on the fringe of poverty and in the middle class" in coping with the additional economic burdens associated with sickle cell disease. He explained that there are various unmeasured financial challenges faced by pediatric patients and their families, such as food and housing insecurity, as well as numerous healthcare expenses. These expenses include seemingly small costs like transportation and parking, which can quickly accumulate.
Tens of thousands of lower-income families with children suffering from sickle cell disease have applied for disability benefits. However, an analysis of federal data obtained through a public records request and shared with CBS News reveals that approximately 76% of these applicants, or at least 12,249 families, had their requests denied between 2015 and 2020.
Medical Experts and Legal Scholars Call for Change in Disability Standards for Children with Sickle Cell Disease
Medical experts and legal scholars are urging for a revision of the disability standards for children with sickle cell disease. They argue that a rule change implemented in 2015 has limited access to benefits for these children. According to these experts, the current standards used by the Social Security Administration (SSA) to determine the severity of disability in children with sickle cell disease are outdated and do not align with the current medical standards for treatment.
Dr. Lewis Hsu, a specialist in pediatric blood disorders at the University of Illinois at Chicago and the chief medical officer for the Sickle Cell Disease Association of America, expressed his confusion with the Social Security disability process. He stated that the disability standards for children with sickle cell disease are "out of date" and lag behind the medical standard by 15 to 20 years.
The 2015 Rule Change
In 2013, the SSA proposed significant changes to the requirements used to evaluate "hematological disorders" such as sickle cell disease. The agency aimed to streamline the guidance for determining eligibility for benefits and how applications would be evaluated. The changes included updates to outdated terminology, clarification on the treatment of specific disorders, and the documentation required from patients to qualify for benefits. The SSA intended for these changes to align with advancements in medical knowledge and their adjudicative experience. The revised criteria took effect in 2015, with adjustments made based on public feedback.
A lawyer from Community Legal Services in Philadelphia, Richard Weishaupt, has expressed concerns over the denial of assistance for young sickle cell patients. Weishaupt, who successfully argued a Supreme Court case on child disability in 1990, stated that there has been a significant increase in denials since a rule change in 2015. In order to obtain nonpublic data on the number of denied benefits between 2011 and 2020, Weishaupt's group filed a public records request with the Social Security Administration (SSA). The results confirmed their suspicions, revealing that the denial rate for child sickle cell cases had risen from an average of 62.3% between 2011 and 2015 to an average of 76% over the following five years. This means that approximately 1,456 children who would have qualified under the old standards were rejected under the new requirements. Weishaupt described the data as "disturbing" and called for a more realistic and fair approach to be implemented.
According to the Social Security Administration (SSA), they have implemented a new formula to determine eligibility for disability benefits. This formula takes into account the frequency and recency of a child's hospitalizations. In order to qualify, a child must have been hospitalized three times in a year, with each stay lasting at least 48 hours and occurring at least 30 days apart. The previous standards only required a single hospital stay related to the disease within the past year of the disability application.
CBS News spoke with Dr. Hsu, a pediatric specialist, who stated that there is no medical basis for the new formula. In fact, doctors are aiming to keep patients out of the hospital and find alternative ways of delivering care, such as intense outpatient care. Dr. Hsu mentioned that as a doctor, his goal is to keep kids out of the hospitals or shorten their stays. However, by doing so, he has inadvertently made some children ineligible for disability benefits, even though they have had a difficult time with their health and education.
The health of a patient's red blood cells also plays a role in determining eligibility for disability benefits. Patients must show that they are anemic, which is determined through a test of hemoglobin levels in their blood. However, modern medications are designed to raise hemoglobin levels, making it more difficult for patients to meet this requirement.
Source: CBS News
"Your functional status remains unchanged, and now you are at risk of losing your Social Security disability benefits, which can be seen as a punishment for receiving modern medical treatment," a concerned individual stated.
The changes made in 2015 also stated that the prescription of opioids could be seen as an indication of potentially disabling pain. However, these strong pain medications come with their own set of risks. The decision to prescribe opioids to children is left to the discretion of each doctor and their patients.
Many of the current concerns regarding the revised standards were brought up during the public comment period, which took place in 2015. Lengthy responses were received, with several comments expressing concern that individuals with hematological disorders may be disabled but not meet the specific medical criteria outlined in the listings. Others disagreed with the criteria itself, including the requirements for hospitalization.
Despite these concerns, the agency largely dismissed them. The Social Security Administration repeatedly referred to "functional criteria" that would allow adjudicators to assess impairments that are difficult to evaluate strictly based on medical terms. These criteria were meant to provide support for individuals who may be severely disabled but do not meet all the medical requirements.
Levella Golden, Heaven's mom, said she was fired from a previous fast food job for calling out too many times to take care of her daughter. Her applications for benefits have been denied five times, and she said she was surprised to see the SSA cite her income in the denials. Some of the rejections said Heaven's medical paperwork didn't reflect the disability standards for sickle cell disease required by SSA, Golden said.
"I'm in the lower class and it's really hard living paycheck to paycheck," she said, pointing to steep grocery bills that come with the healthy, organic diet sickle cell patients require. "If my family was rich, why would we try to get Social Security disability? I don't make enough money."
Other denials stemmed from bureaucratic missteps that left Levella infuriated. After Heaven's extended post-birthday hospital stay, Golden said she met with an SSA administrator in Illinois and asked why they were being shut out.
I asked the man, 'Why are y'all denying her?' He said, 'You signed in the wrong box,'" she recalled. "I felt disrespected. Like, I signed in the wrong box? Are you serious? This is why you all denied her?"
Golden said she asked him where should sign instead.
"And then they still denied Heaven," she said.
Lisa Thaniel is a social worker at Children's National Hospital who oversees the cases of about 1,400 sickle cell disease patients. She said she wishes SSA had more case managers to help parents navigate the application process. Thaniel said parents have "encountered resistance" when contacting SSA. She said she and a patient recently called an SSA phone line and waited for three hours until someone picked up.
SSA does not release denial rates for disability claims by disease, so it is unclear how sickle cell cases compare to other medical issues. However, SSA does automatically approve disability payments for some childhood cancers and other rare disorders.
"A path forward"
Lawmakers have been advocating for government support for sickle cell patients and are concerned about the disability application process. Rep. Barbara Lee, a California Democrat who leads the Congressional Sickle Cell Caucus, is calling for action.
According to an interview with CBS News, Lee expressed concern over the children who are unable to access these payments, stating that it is "borderline immoral and unethical" as it goes against the recommendations of the science and medical profession. Lee also highlighted the disproportionate impact on Black children and called for the SSA to be held accountable.
The responsibility of finding a solution may now rest on the shoulders of Martin O'Malley, the newly confirmed leader of the SSA. O'Malley is the agency's first permanent leader in two and a half years. The SSA has faced challenges in retaining workers and consistently ranks poorly in employee surveys.
While Congress could potentially intervene to adjust how the SSA considers applications, experts believe that any changes or fixes are more likely to come from within the agency itself. Weishaupt, an attorney for Community Legal Services, has urged the SSA to review the standards for sickle cell disease more frequently, similar to other types of disorders.
The current standards for sickle cell disease were initially approved for a five-year period in 2015, with the SSA committing to monitoring and ensuring that they align with program purposes. These standards have since been extended until 2026.
According to a spokesperson for the Social Security Administration (SSA), the agency regularly reviews the requirements for disability benefits related to hematological disorders, including sickle cell disease. The spokesperson stated that the SSA conducts research and updates its listings to account for the impacts, outcomes, and advancements in treatment for these disorders. The SSA also collaborates with medical experts and the Department of Health and Human Services to establish these rules.
The spokesperson further emphasized that the SSA takes lawmakers' concerns seriously and is committed to addressing any issues. In a Senate hearing, Senator O'Malley pledged to work towards removing barriers for children with disabilities in the SSA system.
Recently, the Food and Drug Administration approved a groundbreaking gene-editing treatment for sickle cell disease. This treatment has the potential to repair the gene responsible for the disease.
In Chicago, a mother named Levella continues to apply for disability benefits to support her child, Heaven, who has sickle cell disease. However, she anticipates another denial from the SSA, despite Heaven's increasing hospitalizations. Levella expressed her frustration with the SSA's repeated denials, stating that it feels like a "slap in the face" to be encouraged to keep applying while facing continual rejections. Despite the challenges, Levella remains determined to stay strong for her child.
